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Forniceal Astrocytoma
Joseph Junewick, MD FACR
over 6 years ago
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Case History


Teenager with lethargy.


Case Detail

Anatomy: Gastrointestinal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 7 years ago
Updated: over 7 years ago
Tags: PEDS
Modality/Study Types: CR
Activities:
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Reveal Clinical Notes

History of Hunter disease.

Reveal Findings

CR – Supine and prone cross-table lateral abdominal radiographs reveal marked gaseous distention of large and small bowel with absence of gas in the rectum. Note the presence of gastrostomy tube and ventriculoperitoneal shunt. Also L1 vertebral body is hypoplastic with anterior beaking, the ribs are broad and oar-shaped, the iliac wings are flared and the femoral heads are dysplastic related to dysostosis multiplex (carbohydrate storage disease).

Contrast Enema – Decompressed distal colon with beaking of the column at the point of obstruction.

Reveal Discussion

Twisting of the colon around a dense adhesion was found at surgery. Sigmoid volvulus is unusual in children. It is often seen in those with abnormal mesenteric attachments, neurologic impairment and chronic constipation. On radiography, sigmoid volvulus can be diagnosed as a dilated horseshoe shaped loop of bowel extending out of the pelvis into the upper abdomen (coffee bean sign or northern exposure sign). On contrast enema, beak-like obstruction of the contrast column is characteristic. Treatment can be conservative (rectal tube decompression) if the obstruction is incomplete, otherwise surgery may be indicated.

Hunter disease is phenotypically similar but less severe compared to other mucopolysaccharidoses. Genetic transmission is X-linked recessive (Xq27-q28) and therefore usually only males are affected. The enzymatic defect is of the lysosomal enzyme iduronate sulfatase. Mental retardation is variable.

Reveal Diagnosis

Sigmoid Volvulus

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